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A 24 year old woman with vision loss
Digital Journal of Ophthalmology 2004
Volume 10, Number 10
October 16, 2004
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Kashif Qureshi | Moorfields Eye Hospital
Shahram Kashani | King's College Hospital
Michael Toma | QE2 Hospital, Welwyn Garden City
Diagnosis and Discussion
Susac Syndrome is a microangiopathy of the brain, retina, and cochlea, affecting women aged 21 to 41 years. It follows a 1 – 2 year clinical course, remitting after 2 years. Most improve, but 50% have gait disturbances and dementia. Some develop longstanding visual and hearing loss.

Branch retinal artery occlusions occur, which are invariably bilateral, often as the presenting sign. Posterior pole disease causes visual loss. ESR is often elevated in these patients.

Encephalopathy, which can be subacute, seizures and myoclonus occurs. Encephalopathy may manifest as personality change, bizarre behaviour and paranoia. Bilateral extensor plantar reflexes and pseudobulbar speech patterns are noted.

Hearing loss often occurs associated with tinnitus. Microinfarction of the cochlea causes low to moderate frequency hearing loss. Microinfarction of the labyrinth causes jerk nystagmus.

EEG shows a slow pattern in encephalopathy, and MRI (imaging study of choice) shows multiple infarcts in white and grey matter. CSF protein is elavated on lumbar puncture.

Treatment may consist of intravenous immunoglbulins, steroids, plasma exchange, cyclophosphamide, and hyperbaric oxygen depending on the severity of the symptoms.